Krebs cycle disorders

McCommis KS, Finck BN. Mitochondrial pyruvate transport: a historical perspective and future research directions. Biochem J. 2015 Mar 15;466(3):443–54. PMID: 25748677; PMCID: PMC4464838.

Metabolites

(S)-Methylmalonyl-CoA

Formula: C25H40N7O19P3S (867.131248)

CAS ID: 104809-02-1

ADP

Formula: C10H15N5O10P2 (427.02941500000003)

CAS ID: 58-64-0

ATP

Formula: C10H16N5O13P3 (506.9957476)

CAS ID: 56-65-5

Fumarate

Formula: C4H4O4 (116.01095839999999)

CAS ID: 110-17-8

L-Alanine

Formula: C3H7NO2 (89.0476762)

CAS ID: 56-41-7

Methylmalonate

Formula: C4H6O4 (118.0266076)

CAS ID: 516-05-2

Succinyl-CoA

Formula: C25H40N7O19P3S (867.131248)

CAS ID: 604-98-8

2-oxoglutarate(2-)

Formula: C5H4O5 (144.00587339999998)

CAS ID: 64-15-3

succinate(2-)

Formula: C4H4O4 (116.01095839999999)

CAS ID: 56-14-4

pyruvate

Formula: C3H3O3 (87.00821880000001)

CAS ID: 57-60-3

lactate

Formula: C3H5O3 (89.02386800000001)

CAS ID: 113-21-3



Enzyme

EC Number name full name note
1.2.4.-
1.1.1.27 L-lactate dehydrogenase (S)-lactate:NAD+ oxidoreductase
1.2.4.2 oxoglutarate dehydrogenase (succinyl-transferring) 2-oxoglutarate:[dihydrolipoyllysine-residue succinyltransferase]-lipoyllysine 2-oxidoreductase (decarboxylating, acceptor-succinylating)
2.2.1.5 2-hydroxy-3-oxoadipate synthase 2-oxoglutarate:glyoxylate succinaldehydetransferase (decarboxylating)
2.3.1.61 dihydrolipoyllysine-residue succinyltransferase succinyl-CoA:enzyme-N6-(dihydrolipoyl)lysine S-succinyltransferase
2.6.1.2 alanine transaminase L-alanine:2-oxoglutarate aminotransferase
4.1.1.71 2-oxoglutarate decarboxylase 2-oxoglutarate carboxy-lyase (succinate-semialdehyde-forming)
6.2.1.4 succinate-CoA ligase (GDP-forming) succinate:CoA ligase (GDP-forming)
6.2.1.5 succinate-CoA ligase (ADP-forming) succinate:CoA ligase (ADP-forming)
6.2.1.9 malate-CoA ligase malate:CoA ligase (ADP-forming)


Proteins

Protein ID name full name
Q1B4V6 kgd Multifunctional 2-oxoglutarate metabolism enzyme